Two brothers with rare blood disease appeal for help

Two brothers with rare blood disease appeal for help
By  Willie Ong
Wednesday, July 16, 2008

Philippine Star

A mother of six, Lorena de Guzman comes from a poor family in Bulacan. Two of her children, Gian, 13, and eight-year-old Rhael have Thalassemia Major, a rare blood disorder.

The De Guzmans live at 077 Cacarong Matanda, Pandi, Bulacan. Lorena is a housewife and father Carlito is an embroiderer. For the past eight years, Lorena has approached practically all available government and non-government options to prolong the life of her sons. A friend suggested she approach The Philippine STAR’s socio-humanitarian arm, Operation Damayan.

What is Thalassemia?

Thalassemia is an inherited disorder of the blood. Depending on the number of abnormal genes, patients can have mild, intermediate or severe form of Thalassemia. Brothers Gian and Rhael suffer from the most severe type called Thalassemia Major or Cooley’s Anemia. In a survey in the United States , only 513 Americans have this disease.

Patients with this condition completely lack a certain protein in their hemoglobin. And since hemoglobin is part of the normal red blood cells of the body, these patients develop severe anemia.

Symptoms start to occur at two years of age when the patients develop fatigue, difficulty of breathing, paleness and dark urine. If untreated, patients develop heart failure or infections leading to early death. Diagnosis is by simple blood tests.

The usual treatments for Thalassemia Major are regular blood transfusion, medications and spleen removal. Complications are common with frequent blood transfusion because patients develop iron-overload. Close medical follow-up is needed.

Starting at age five, Gian and Rhael have been undergoing monthly blood transfusions at the National Children’s Hospital worth P5,000 per month each. As a result, both Gian and Rhael have dark gray and stippled faces from too much iron in their blood. Their whole bodies, internal organs and skin, are stained dark ashen gray.

Upon examination, Gian’s latest Serum Iron levels are at 5515 ng/ml, which is 15 times higher than a normal person. As a result, Gian’s spleen has enlarged tremendously, giving him a protruding belly.

How long will they live?

According to medical science, the prognosis of Thalassemia Major is not good. Some patients live only until their teens. With the best care, they can live up to adulthood. Gian would also need to undergo a spleen removal surgery later on to prolong his life.

But despite his weakened condition, there is something so innocent and sweet about Gian.

In their Bulacan home, Gian loves to care for wayward animals: a goat, two dogs, six pigeons, 12 native chickens and a guinea pig. Gian then sells the chicken for P100 each and gives all the money to his mother.

For Gian, as long as his mom is there, he is safe. In return, Lorena’s only wish is to have her two sons live a bit longer and with some comfort.

The brothers need around P10,000 a month to have a fighting chance at life. This amount will meet their medical expenses and help prolong their lives.

For those who would like to help Gian and Rhael, you may contact their mother Lorena at cell phone number 0916-377-7969. You may also donate through Operation Damayan at telephone number 527-7901.

Lorena would like to thank the people who have helped her children: Ms. Stephanie Zallow, a German nurse, Bulacan Gov. Mendoza, Mr. Daniel Razon, Dr. Bernie dela Cruz of the Quezon City Red Cross, One Pharma, and the kind personnel and doctors of the National Children’s Hospital.

E-mail questions to drwillieong–@gmail.com